scholarly journals Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

2020 ◽  
Author(s):  
Yingjie Xu ◽  
Xiaolin Ma ◽  
Zhixuan Zhou ◽  
Jianguo Li ◽  
jun Huo ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Early Diagnosis ◽  
Juvenile Dermatomyositis ◽  
Case Reports ◽  
Relevant Literature ◽  
Ureteral Stricture ◽  
Ureteral Calculus ◽  
Intermittent Fever ◽  
Fatal Complication ◽  
Initial Symptoms

Abstract Background: To summarize the characteristics of gastrointestinal (GI) perforation in anti-NXP2 antibody-associated juvenile dermatomyositis (JDM). Methods: Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed. Results: Five patients, including 4 females and 1 male, were involved in the study. The average age of the 5 patients was 5.74 years (5.74 ±2.82). Myositis-specific antibody (MAS) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients, which was successful in only one patient through fistulization. Four patients died (4/5, 80%), and 1 patient was completely relieved after 2 years of treatment. Combined with 16 patients described in the literature, the onset symptom was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most often occurred in the duodenum, although perforations at multiple sites or recurrent perforations also occurred. The mortality rate of GI perforation in JDM was 38% (8/21). Conclusions: All perforation cases subjected to MAS analysis were anti-NXP2 antibody positive. The onset symptom was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations complicated early diagnosis. GI perforation is a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

scholarly journals Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

2020 ◽  
Author(s):  
Yingjie Xu ◽  
Xiaolin Ma ◽  
Zhixuan Zhou ◽  
Jianguo Li ◽  
jun Huo ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Early Diagnosis ◽  
Matrix Protein ◽  
Age Of Onset ◽  
Juvenile Dermatomyositis ◽  
Case Reports ◽  
The Other ◽  
Ureteral Stricture ◽  
Ureteral Calculus ◽  
Initial Symptoms

Abstract Background: To summarize the characteristics of gastrointestinal (GI) perforation in anti-nuclear matrix protein 2 (NXP2) antibody-associated juvenile dermatomyositis (JDM). Methods: Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed.Results: Five patients, including four females and one male, were included in the study. The age of onset of these patients ranged from 3.3 to 9.5 years with the median age of 5.0 years. When these patients were complicated by GI perforation, childhood myositis assessment score (CMAS) ranged from 1 to 5 with the median score of 2. Myositis-specific antibody (MSA) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients. One patient failed to undergo a repair as the perforation was high in position. For the other three patients, perforation repair was successful, of which two patients failed due to recurrent perforation. At 24 months postoperative follow-up, one patient was in complete remission on prednisone (Pred) and methotrexate(MTX) treatment, and her ureteral stricture had disappeared. The other four patients died. Adding these cases with 16 other patients described in the literature, the symptom at onset was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most commonly occurred in the duodenum, although it also occurred at multiple sites or was recurrent. The mortality rate of GI perforation in JDM was 38% (8/21). Conclusions: All the five perforation cases in our study subjected to MSA analysis were anti-NXP2 antibody positive. The symptom at onset was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations prevented early diagnosis. GI perforation may be a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

scholarly journals Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: case reports and a review of the literature

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Yingjie Xu ◽  
Xiaolin Ma ◽  
Zhixuan Zhou ◽  
Jianguo Li ◽  
Jun Hou ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Early Diagnosis ◽  
Matrix Protein ◽  
Age Of Onset ◽  
Juvenile Dermatomyositis ◽  
Case Reports ◽  
The Other ◽  
Ureteral Stricture ◽  
Ureteral Calculus ◽  
Initial Symptoms

Abstract Background To summarize the characteristics of gastrointestinal (GI) perforation in anti-nuclear matrix protein 2 (NXP2) antibody-associated juvenile dermatomyositis (JDM). Methods Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed. Results Five patients, including four females and one male, were included in the study. The age of onset of these patients ranged from 3.3 to 9.5 years with the median age of 5.0 years. When these patients were complicated by GI perforation, childhood myositis assessment score (CMAS) ranged from 1 to 5 with the median score of 2. Myositis-specific antibody (MSA) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients. One patient failed to undergo a repair as the perforation was high in position. For the other three patients, perforation repair was successful, of which two patients failed due to recurrent perforation. At 24 months postoperative follow-up, one patient was in complete remission on prednisone (Pred) and methotrexate (MTX) treatment, and her ureteral stricture had disappeared. The other four patients died. Adding these cases with 16 other patients described in the literature, the symptom at onset was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most commonly occurred in the duodenum, although it also occurred at multiple sites or was recurrent. The mortality rate of GI perforation in JDM was 38% (8/21). Conclusions All the five perforation cases in our study subjected to MSA analysis were anti-NXP2 antibody positive. The symptom at onset was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations prevented early diagnosis. GI perforation may be a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

scholarly journals Postcolonoscopy splenic rupture: the under-reporting of an unpropitious phenomena?

2019 ◽  
Vol 12 (9) ◽  
pp. e231047 ◽  
Author(s):  
Bing Lun Chow ◽  
Khawaja Zia
Keyword(s):  
Abdominal Pain ◽  
Case Report ◽  
Chest Pain ◽  
Ct Scan ◽  
Splenic Rupture ◽  
Case Reports ◽  
Initial Presentation ◽  
Haemodynamic Instability ◽  
Fatal Complication

Splenic rupture secondary to colonoscopy is a rare but potentially fatal complication. Given the disparity between the small number of case reports with the incidence reported by some investigators, we contend that the former is not representative of the true extent of this sequela. We present a case report of postcolonoscopy splenic rupture, where the patient had a bizarre initial presentation of chest pain and collapse; and only developed haemodynamic instability and abdominal pain on day 2 postprocedure. Diagnosis was made with a CT scan, and resolution of symptoms was achieved with a splenectomy.

scholarly journals Interferon-induced sarcoidosis with uveitis as the initial symptom: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ai Kato ◽  
Mami Ishihara ◽  
Nobuhisa Mizuki
Keyword(s):  
Case Reports ◽  
Relevant Literature ◽  
Corticosteroid Treatment ◽  
Pegylated Interferon ◽  
Skin Lesions ◽  
Japanese Woman ◽  
Interferon Α ◽  
Initial Symptom ◽  
Initial Symptoms ◽  
Old Japanese

Abstract Background In recent years, numerous studies have reported the development or exacerbation of sarcoidosis due to interferon therapy. However, ocular lesions rarely present as initial symptoms. Herein, we describe a rare case of interferon-α-induced sarcoidosis with uveitis as the initial symptom, and present a review of the relevant literature. Case presentation This case involved a 62-year-old-Japanese woman with a history of a combination treatment of pegylated interferon-α-2a, ribavirin, and simeprevir, after which she developed granulomatous panuveitis. She was subsequently diagnosed with sarcoidosis following histological examination of skin biopsy specimens. In addition to reporting this case, we performed a literature review of 27 cases (24 case reports) of histopathologically diagnosed interferon-α-induced sarcoidosis published between January 2009 and November 2018. Conclusions Among the reviewed cases, 23 (85.1%) cases developed skin lesions and 19 (70.1%) had lung lesions. Only three cases (11.1%) had accompanying eye lesions. Interferon-α therapy was discontinued in 16 cases (52.9%), and the majority exhibited improvement after systemic corticosteroid treatment. There are few reported cases of interferon-α-induced sarcoidosis with uveitis as the initial symptom. However, if uveitis develops during or after interferon-α treatment, it might represent an initial symptom of interferon-α-induced sarcoidosis, as observed in the present case.

Hernia interna estrangulada por plastrón en el tercio distal del apéndice cecal

2019 ◽  
Vol 62 (6) ◽  
pp. 24-27
Author(s):  
Leslie M. Leyva Sotelo ◽  
José E. Telich Tarriba ◽  
Daniel Ángeles Gaspar ◽  
Osvaldo I. Guevara Valmaña ◽  
André Víctor Baldín ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Small Bowel ◽  
Early Diagnosis ◽  
Intestinal Obstruction ◽  
Acute Abdomen ◽  
Internal Hernia ◽  
Wide Spectrum ◽  
Internal Hernias ◽  
Mild Abdominal Pain ◽  
Made In

Internal hernias are an infrequent cause of intestinal obstruction with an incidence of 0.2-0.9%, therefore their early diagnosis represents a challenge. The most frequently herniated organ is the small bowel, which results in a wide spectrum of symptoms, varying from mild abdominal pain to acute abdomen. We present the case of an eight-year old patient with nonspecific digestive symptoms, a transoperative diagnosis was made in which an internal hernia was found strangulated by plastron in the distal third of the appendix. Appendectomy was performed and four days later the patient was discharged without complications.

HYDRAMNIOS AS AN AID TO THE EARLY DIAGNOSIS OF CONGENITAL OBSTRUCTION OF THE ALIMENTARY TRACT: A STUDY OF THE MATERNAL AND FETAL FACTORS

PEDIATRICS ◽  
1958 ◽  
Vol 21 (6) ◽  
pp. 903-909
Author(s):  
James R. Lloyd ◽  
H. William Clatworthy
Keyword(s):  
Early Diagnosis ◽  
Alimentary Tract ◽  
Case Reports ◽  
Perinatal Period ◽  
University Hospital ◽  
Diagnostic Significance ◽  
Children's Hospital ◽  
Obstructive Disease ◽  
Children’S Hospital ◽  
The University

ALTHOUGH the association between hydramnios and congenital anomalies of the alimentary tract of the fetus has been recognized for more than 75 years, little emphasis has been placed upon this prenatal maternal abnormality as an aid to the earlier diagnosis of obstructive disease in the newborn. With the exception of isolated case reports which were summarized by Taussig in 1927 and the more recent observations of Mengert and Bourland, Castanier et al., and Clatworthy and Lloyd, the diagnostic significance of this relationship has not received proper recognition. Fortunately, today, the majority of anatomic abnormalities of the alimentary tract which are encountered in the neonate can be surgically corrected, if the diagnosis can be established and the treatment instituted before the infant has deteriorated irreversibly. A more general appreciation of the importance of hydramnios as a diagnostic sign of obstructive disease by physicians responsible for the care of the infant during the perinatal period should improve infant salvage. Recent surveys of our experience at the Columbus Children's Hospital with neonates having obstructing lesions of the esophagus or intestine attracted our attention to the fact that many of these infants were delivered of mothers who suffered from acute or chronic hydramnios. In an effort to determine the significance of the interrelationship between hydramnios in the mother and congenital obstruction of the alimentary tract in the fetus, a study was undertaken which included not only a survey of all infants admitted to the Children's Hospital with alimentary tract obstruction but also an analysis of a series of obstetric cases at the University Hospital in women with pregnancy complicated by hydramnios.

Ischemic stroke as a presenting manifestation of polycythemia vera: a narrative review

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Marco Burattini ◽  
Lorenzo Falsetti ◽  
Eleonora Potente ◽  
Claudia Rinaldi ◽  
Marco Bartolini ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
Early Diagnosis ◽  
Polycythemia Vera ◽  
Case Reports ◽  
Previous History ◽  
Recurrence Risk ◽  
Narrative Review ◽  
Stroke Recurrence ◽  
Increased Risk

Abstract Polycythemia vera (PV) is a myeloproliferative disorder associated with an increased risk of cerebrovascular diseases. In this narrative review, we aimed to analyze the relationships between acute ischemic stroke and PV. We conducted a PubMed/Medline and Web of Sciences Database search using MeSH major terms. We found 75 articles and finally considered 12 case reports and 11 cohort studies. The ischemic stroke resulted as the first manifestation of PV in up to 16.2% of cases; the cumulative rate of cerebrovascular events was up to 5.5 per 100 persons per year and stroke accounted for 8.8% of all PV-related deaths; age, mutations, and a previous history of thrombosis were the main risk factors. The best approach to reduce stroke recurrence risk is unclear, even if some evidence suggests a potential role of lowering hematocrit below 45%. Ischemic stroke represents one of the most common PV manifestations but, despite their relationship, patients with both diseases have a very heterogeneous clinical course and management. PV-related strokes often remain underdiagnosed, especially for the low prevalence of PV. An early diagnosis could lead to prompt treatment with phlebotomy, cytoreduction, and low-dose aspirin to decrease the risk of recurrences. Clinicians should be aware of PV as a risk factor for stroke when approaching the differential diagnosis of cryptogenic strokes. An early diagnosis could positively influence patients’ management and clinical outcomes. Further studies are required to evaluate the role of PV treatments in the prevention of cerebrovascular disease.

Fulminant varicella hepatitis: a rare but lethal cause of abdominal pain

2021 ◽  
Vol 14 (9) ◽  
pp. e244081
Author(s):  
Christopher Fang ◽  
Junice Wong ◽  
Wei Wen Ang
Keyword(s):  
Abdominal Pain ◽  
Hepatitis A ◽  
Case Reports ◽  
Antibody Testing ◽  
Salient Points ◽  
Vesicular Rash ◽  
History Of ◽  
Laboratory Investigations ◽  
The Common ◽  
Upper Abdominal

An 81-year-old woman with no history of immunocompromise presented with 2 days of upper abdominal pain associated with nausea. On arrival, her physical examination was unremarkable apart from mild epigastric and right hypochondriac tenderness, and laboratory investigations were unremarkable apart from mild thrombocytopenia and transaminitis. A CT scan performed on the day of admission revealed a tiny 0.3 cm stone in the common bile duct, with no upstream dilatation. On day 2 of admission, she developed a vesicular rash and with acutely worsening transaminitis. She deteriorated rapidly and demised from complications of acute liver failure within the next 24 hours. The diagnosis of varicella was confirmed with antibody testing. Fulminant varicella hepatitis is an extremely rare and lethal condition with only a handful of reported cases in the current literature. We aim to share our clinical experience and summarise the salient points from existing case reports.

scholarly journals Next-Generation Sequencing in Early Diagnosis of Dent Disease 1: Two Case Reports

2018 ◽  
Vol 5 ◽  
Author(s):  
Min Wen ◽  
Tian Shen ◽  
Ying Wang ◽  
Yongzhen Li ◽  
Xiaoliu Shi ◽  
...  
Keyword(s):  
Next Generation Sequencing ◽  
Early Diagnosis ◽  
Case Reports ◽  
Dent Disease ◽  
Next Generation ◽  
Generation Sequencing

scholarly journals Acute Pancreatitis: An Atypical Presentation

2017 ◽  
Vol 11 (2) ◽  
pp. 359-363 ◽  
Author(s):  
Omar Nadhem ◽  
Omar Salh
Keyword(s):  
Acute Pancreatitis ◽  
Abdominal Pain ◽  
Case Reports ◽  
Case Series ◽  
Normal Serum ◽  
Intravenous Fluids ◽  
Serum Enzyme ◽  
Serum Lipase ◽  
Upper Abdominal ◽  
Symptoms And Signs

Acute pancreatitis is an important cause of acute upper abdominal pain. Because its clinical features are similar to a number of other acute illnesses, it is difficult to make a diagnosis only on the basis of symptoms and signs. The diagnosis of acute pancreatitis is based on 2 of the following 3 criteria: (1) abdominal pain consistent with pancreatitis, (2) serum lipase and/or amylase ≥3 times the upper limit of normal, and (3) characteristic findings from abdominal imaging. The sensitivity and specificity of lipase in diagnosing acute pancreatitis are undisputed. However, normal lipase level should not exclude a pancreatitis diagnosis. In patients with atypical pancreatitis presentation, imaging is needed. We experienced two cases of acute pancreatitis associated with normal serum enzyme levels. Both patients were diagnosed based on clinical and radiological evidence. They were successfully treated with intravenous fluids and analgesics with clinical and laboratory improvement. The importance of this case series is the unlikely presentation of acute pancreatitis. We believe that more research is needed to determine the exact proportion of acute pancreatitis patients who first present with normal serum lipase, since similar cases have been seen in case reports.

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